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FITC标记的伴侣蛋白bc1同源复合体抗体

2017-5-2


                                    FITC标记的伴侣蛋白bc1同源复合体抗体                                                                                                                                                
英文名称Anti-CABC1/FITC
中文名称:FITC标记的伴侣蛋白bc1同源复合体抗体
别    名mitochondrial; aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; ADCK3; ADCK3_HUMAN; CABC 1; Chaperone ABC1 (activity of bc1 complex S.pombe) like; Chaperone ABC1 activity of bc1 complex homolog; Chaperone ABC1 like; Chaperone activity of bc1 complex like; Chaperone activity of bc1 complex like mitochondrial; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q8 homolog; COQ 8; COQ8.  

详细介绍:


规格:100ul 
说 明 书100ul  
研究领域肿瘤  细胞生物  神经生物学  信号转导  线粒体  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量72kDa
性    状Lyophilized or Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ADCK3/CABC1
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

相关资料:


产品介绍background:
This gene encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. It is not related to the family of ABC transporter proteins. Expression of this gene is induced by the tumor suppressor p53 and in response to DNA damage, and inhibiting its expression partially suppresses p53-induced apoptosis. Alternatively spliced transcript variants have been found; however, their full-length nature has not been determined. [provided by RefSeq, Jul 2008]

Function:
May be a chaperone-like protein essential for the properconformation and functioning of protein complexes in therespiratory chain. 

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Ubiquitously expressed with a relativelygreater abundance in heart and skeletal muscle.

DISEASE:
Defects in ADCK3 are the cause of coenzyme Q10deficiency, primary, type 4 (COQ10D4) [MIM:612016]. An autosomalrecessive disorder characterized by childhood-onset of cerebellarataxia and exercise intolerance. Patient manifest gait ataxia andcerebellar atrophy with slow progression. Additional featuresinclude brisk tendon reflexes and Hoffmann sign, variablepsychomotor retardation and variable seizures.

Similarity:
Belongs to the protein kinase superfamily. ADCKprotein kinase family.
Contains 1 protein kinase domain.

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